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      The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy

      review-article
      , MD,PhD 1 , , , MD,PhD 2 , , , MD,PhD 3 , , MD,PhD 1 , , MD,PhD 3 , 4 , , MD,PhD 1 , , MD,PhD 5 , , MD,PhD 6 , 7 , , MD,PhD 1 , , MD,PhD 8 , 9 , , MD,PhD 3 , 4 , , MD 10 , , MD 11 , , MD,PhD 12 , 13 , , MD 14 , , MD,PhD 15 , 16 , , MD 17 , , MD 18 , , MD,PhD 15 , 19 , , MD,FRCP 20 , 21 , , MD,PhD 22 , , MD,FRCP 20 , , MD,PhD 23 , , MD,PhD 24 , 25 , , MD,PhD 26 , 27 , , MD,PhD 28 , 29 , , MD,PhD 15 , , MD 1 , , MD 5 ,
      Movement Disorders
      John Wiley & Sons, Inc.
      multiple system atrophy, diagnostic criteria, diagnosis

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          ABSTRACT

          Background

          The second consensus criteria for the diagnosis of multiple system atrophy (MSA) are widely recognized as the reference standard for clinical research, but lack sensitivity to diagnose the disease at early stages.

          Objective

          To develop novel Movement Disorder Society (MDS) criteria for MSA diagnosis using an evidence‐based and consensus‐based methodology.

          Methods

          We identified shortcomings of the second consensus criteria for MSA diagnosis and conducted a systematic literature review to answer predefined questions on clinical presentation and diagnostic tools relevant for MSA diagnosis. The criteria were developed and later optimized using two Delphi rounds within the MSA Criteria Revision Task Force, a survey for MDS membership, and a virtual Consensus Conference.

          Results

          The criteria for neuropathologically established MSA remain unchanged. For a clinical MSA diagnosis a new category of clinically established MSA is introduced, aiming for maximum specificity with acceptable sensitivity. A category of clinically probable MSA is defined to enhance sensitivity while maintaining specificity. A research category of possible prodromal MSA is designed to capture patients in the earliest stages when symptoms and signs are present, but do not meet the threshold for clinically established or clinically probable MSA. Brain magnetic resonance imaging markers suggestive of MSA are required for the diagnosis of clinically established MSA. The number of research biomarkers that support all clinical diagnostic categories will likely grow.

          Conclusions

          This set of MDS MSA diagnostic criteria aims at improving the diagnostic accuracy, particularly in early disease stages. It requires validation in a prospective clinical and a clinicopathological study. © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society

          Abstract

          MDS Criteria for the Diagnosis of Multiple System Atrophy

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          Most cited references84

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          MDS clinical diagnostic criteria for Parkinson's disease.

          This document presents the Movement Disorder Society Clinical Diagnostic Criteria for Parkinson's disease (PD). The Movement Disorder Society PD Criteria are intended for use in clinical research but also may be used to guide clinical diagnosis. The benchmark for these criteria is expert clinical diagnosis; the criteria aim to systematize the diagnostic process, to make it reproducible across centers and applicable by clinicians with less expertise in PD diagnosis. Although motor abnormalities remain central, increasing recognition has been given to nonmotor manifestations; these are incorporated into both the current criteria and particularly into separate criteria for prodromal PD. Similar to previous criteria, the Movement Disorder Society PD Criteria retain motor parkinsonism as the core feature of the disease, defined as bradykinesia plus rest tremor or rigidity. Explicit instructions for defining these cardinal features are included. After documentation of parkinsonism, determination of PD as the cause of parkinsonism relies on three categories of diagnostic features: absolute exclusion criteria (which rule out PD), red flags (which must be counterbalanced by additional supportive criteria to allow diagnosis of PD), and supportive criteria (positive features that increase confidence of the PD diagnosis). Two levels of certainty are delineated: clinically established PD (maximizing specificity at the expense of reduced sensitivity) and probable PD (which balances sensitivity and specificity). The Movement Disorder Society criteria retain elements proven valuable in previous criteria and omit aspects that are no longer justified, thereby encapsulating diagnosis according to current knowledge. As understanding of PD expands, the Movement Disorder Society criteria will need continuous revision to accommodate these advances.
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            Second consensus statement on the diagnosis of multiple system atrophy.

            A consensus conference on multiple system atrophy (MSA) in 1998 established criteria for diagnosis that have been accepted widely. Since then, clinical, laboratory, neuropathologic, and imaging studies have advanced the field, requiring a fresh evaluation of diagnostic criteria. We held a second consensus conference in 2007 and present the results here. Experts in the clinical, neuropathologic, and imaging aspects of MSA were invited to participate in a 2-day consensus conference. Participants were divided into five groups, consisting of specialists in the parkinsonian, cerebellar, autonomic, neuropathologic, and imaging aspects of the disorder. Each group independently wrote diagnostic criteria for its area of expertise in advance of the meeting. These criteria were discussed and reconciled during the meeting using consensus methodology. The new criteria retain the diagnostic categories of MSA with predominant parkinsonism and MSA with predominant cerebellar ataxia to designate the predominant motor features and also retain the designations of definite, probable, and possible MSA. Definite MSA requires neuropathologic demonstration of CNS alpha-synuclein-positive glial cytoplasmic inclusions with neurodegenerative changes in striatonigral or olivopontocerebellar structures. Probable MSA requires a sporadic, progressive adult-onset disorder including rigorously defined autonomic failure and poorly levodopa-responsive parkinsonism or cerebellar ataxia. Possible MSA requires a sporadic, progressive adult-onset disease including parkinsonism or cerebellar ataxia and at least one feature suggesting autonomic dysfunction plus one other feature that may be a clinical or a neuroimaging abnormality. These new criteria have simplified the previous criteria, have incorporated current knowledge, and are expected to enhance future assessments of the disease.
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              A guide for the design and conduct of self-administered surveys of clinicians.

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                Author and article information

                Contributors
                gregor.wenning@i-med.ac.at
                idstanko139@gmail.com
                horacio.kaufmann@nyulangone.org
                Journal
                Mov Disord
                Mov Disord
                10.1002/(ISSN)1531-8257
                MDS
                Movement Disorders
                John Wiley & Sons, Inc. (Hoboken, USA )
                0885-3185
                1531-8257
                21 April 2022
                June 2022
                : 37
                : 6 ( doiID: 10.1002/mds.v37.6 )
                : 1131-1148
                Affiliations
                [ 1 ] Department of Neurology Innsbruck Medical University Innsbruck Austria
                [ 2 ] Neurology Clinic, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade Belgrade Serbia
                [ 3 ] IRCCS, Istituto delle Scienze Neurologiche di Bologna Bologna Italy
                [ 4 ] Department of Biomedical and Neuromotor Sciences University of Bologna Bologna Italy
                [ 5 ] Department of Neurology, Dysautonomia Center, Langone Medical Center New York University School of Medicine New York New York USA
                [ 6 ] French Reference Center for MSA, Department of Neurology for Neurodegenerative Diseases University Hospital Bordeaux, 33076 Bordeaux and Institute of Neurodegenerative Diseases, University Bordeaux, CNRS Bordeaux France
                [ 7 ] Department of Medicine University of Otago, Christchurch, and New Zealand Brain Research Institute Christchurch New Zealand
                [ 8 ] Department of Neurodegeneration and Hertie‐Institute for Clinical Brain Research University of Tübingen Tübingen Germany
                [ 9 ] Department of Neurology Christian‐Albrechts‐University Kiel Kiel Germany
                [ 10 ] Department of Neurology, Beth Israel Deaconess Medical Center Harvard Medical School Boston Massachusetts USA
                [ 11 ] Brain and Mind Centre, Faculty of Medicine and Health School of Medical Sciences, The University of Sydney Sydney New South Wales Australia
                [ 12 ] Department of Neurology Hanover Medical School Hanover Germany
                [ 13 ] German Center for Neurodegenerative Diseases Munich Germany
                [ 14 ] Edmond J. Safra Program in Parkinson's Disease University Health Network and the Division of Neurology, University of Toronto Toronto Canada
                [ 15 ] Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology London United Kingdom
                [ 16 ] Reta Lila Weston Institute of Neurological Studies UCL Queen Square Institute of Neurology London United Kingdom
                [ 17 ] Department of Neurosciences Parkinson and Other Movement Disorders Center, University of California San Diego California USA
                [ 18 ] Department of Neurology Mayo Clinic Rochester Minnesota USA
                [ 19 ] Department of Neuropathology Institute of Brain Science, Hirosaki University Graduate School of Medicine Hirosaki Japan
                [ 20 ] UCL Queen Square Institute of Neurology London United Kingdom
                [ 21 ] Department of Uro‐Neurology The National Hospital for Neurology and Neurosurgery, Queen Square London United Kingdom
                [ 22 ] Department of Medicine Surgery and Dentistry “Scuola Medica Salernitana”, Neuroscience Section, University of Salerno Salerno Italy
                [ 23 ] Neurology, Internal Medicine Sakura Medical Center, Toho University Sakura Japan
                [ 24 ] Parkinson's Disease and Movement Disorders Department HYGEIA Hospital, and Aiginiteion Hospital, University of Athens Athens Greece
                [ 25 ] Philipps University Marburg, Germany and European University of Cyprus Nicosia Cyprus
                [ 26 ] Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED) Hospital Clínic, IDIBAPS, Universitat de Barcelona Catalonia Spain
                [ 27 ] Movement Disorders Unit, Neurology Service Hospital Clínic de Barcelona Catalonia Spain
                [ 28 ] Department of Molecular Neurology The University of Tokyo, Graduate School of Medicine Tokyo Japan
                [ 29 ] International University of Health and Welfare Chiba Japan
                Author notes
                [*] [* ] Correspondence to: Prof. Dr. Gregor K. Wenning, Division of Clinical Neurobiology, Department of Neurology, Medical University Innsbruck, Anichstrasse 35, A‐6020 Innsbruck, Austria; E‐mail: gregor.wenning@ 123456i-med.ac.at ; Prof. Dr. Horacio Kaufmann, Department of Neurology, Dysautonomia Center, New York University School of Medicine, New York, NY, USA; E‐mail: horacio.kaufmann@ 123456nyulangone.org ; Dr. Iva Stankovic, Neurology Clinic, University Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, Serbia; E‐mail: idstanko139@ 123456gmail.com

                Author information
                https://orcid.org/0000-0003-1484-4717
                https://orcid.org/0000-0002-9051-7091
                https://orcid.org/0000-0002-2854-4179
                https://orcid.org/0000-0001-6503-1455
                https://orcid.org/0000-0002-1345-6774
                https://orcid.org/0000-0003-2172-7527
                https://orcid.org/0000-0002-4493-5073
                https://orcid.org/0000-0002-3633-8818
                https://orcid.org/0000-0003-0422-8398
                https://orcid.org/0000-0001-7587-6187
                https://orcid.org/0000-0003-1532-3526
                https://orcid.org/0000-0002-5803-169X
                https://orcid.org/0000-0003-1668-9925
                https://orcid.org/0000-0002-1851-9981
                Article
                MDS29005
                10.1002/mds.29005
                9321158
                35445419
                91d7cb2e-2941-4762-b366-e3d1a292926e
                © 2022 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                : 25 January 2022
                : 10 November 2021
                : 28 February 2022
                Page count
                Figures: 2, Tables: 4, Pages: 18, Words: 13586
                Categories
                Review
                Regular Issue Articles
                Reviews
                Custom metadata
                2.0
                June 2022
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.1.7 mode:remove_FC converted:26.07.2022

                Medicine
                multiple system atrophy,diagnostic criteria,diagnosis
                Medicine
                multiple system atrophy, diagnostic criteria, diagnosis

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